Neuroblastoma: solving a biologic puzzle.

Abstract

Neuroblastoma, a childhood tumor arising in the adrenal medulla and sympathetic nervous system, presents an unusual challenge to pediatric oncologists. While virtually all patients with localized neuroblastoma can be cured by surgery alone, and most infants with disseminated disease are curable with chemotherapy, children with bone metastases usually have a fatal outcome [1–4]. Furthermore, in some patients the tumor undergoes spontaneous regression or differentiation [5,6]. This wide range of clinical behavior reflects the biologic heterogeneity of neuroblastoma. Much progress has been made recently in advancing our knowledge of human neuroblastoma at the cellular and molecular level, and specific genetic abnormalities have been identified that are characteristic of subsets of these tumors [7]. These transformation-linked genetic changes have contributed to our understanding of tumor predisposition, metastasis, treatment responsiveness, and prognosis. Continued study of these genetic abnormalities will hopefully lead to further insight into the molecular events associated with the malignant transformation of this heterogeneous tumor, and thereby provide clues for the development of specific therapy.